Collagen Type IV Alpha 3 Binding Protein (COL4a3BP)

COL4A3BP encodes a kinase that specifically phosphorylates the N-terminal region of the non-collagenous domain of the alpha 3 chain of type IV collagen, known as the Goodpasture antigen. Goodpasture disease is the result of an autoimmune response directed at this antigen. One isoform of this protein is also involved in ceramide intracellular transport. Two transcripts exist for this gene.

Sequence analysis revealed that 18% of the GPBP residues are phosphorylatable and 16% are acidic. Serine residues comprise 9% of the protein, but in 2 regions account for 40% of the amino acids. There is also a pleckstrin homology domain at the N terminus.